title: Hemophagocytic lymphohistiocytosis (HLH): a review of literature.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease in children and an exceptionally rare occurrence in adults. It is categorized broadly into primary (familial) or the secondary types; the latter being associated most commonly with an underlying malignancy. HLH carries a high rate of mortality, and the treatment itself is associated with significant morbidity and risk of mortality. A high degree of suspicion for the diagnosis, early treatment and aggressive supportive care is critical for management. We present a comprehensive review of literature describing the clinical features, diagnosis, management and outcome of HLH.
authors.affiliation: Division of Hematology-Oncology, University of Pittsburgh Medical Center, UPMC Cancer Pavilion, Room 463, 5150 Centre Avenue, Pittsburgh, PA, 15232, USA, mehtars@upmc.edu.
authors.forename: Rohtesh S
authors.initials: RS
authors.lastname: Mehta
authors.forename: Roy E
authors.initials: RE
authors.lastname: Smith
mesh_terms: D019468:Disease Management; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*